Association of idiopathic hepatic sinusoidal dilatation with the immunological features of the antiphospholipid syndrome
- D Saadoun1,
- D Cazals-Hatem2,
- M-H Denninger3,
- L Boudaoud3,
- B-N Pham3,
- V Mallet1,
- B Condat1,
- J Brière4,
- D Valla1
- 1Service d’Hépatologie and INSERM U 481, Hôpital Beaujon (Assistance Publique-Hôpitaux de Paris, Université Paris 7), Clichy, France
- 2Service d’Anatomie et de Cytologie Pathologiques, Hôpital Beaujon (Assistance Publique-Hôpitaux de Paris, Université Paris 7), Clichy, France
- 3Service d’Hématologie et d’Immunologie, Hôpital Beaujon (Assistance Publique-Hôpitaux de Paris, Université Paris 7), Clichy, France
- 4Service d’Hématologie Clinique, Hôpital Beaujon (Assistance Publique-Hôpitaux de Paris, Université Paris 7), Clichy, France
- Correspondence to:
Professor D Valla
Service d’Hépatologie, Hôpital Beaujon, 92118 Clichy, France;
- Received 5 February 2004
- Accepted 30 March 2004
Background: Isolated sinusoidal dilatation is an uncommon hepatic lesion and the cause is largely unknown.
Objective: To investigate whether prothrombotic disorders or perisinusoidal cell changes could be involved in pure idiopathic hepatic sinusoidal dilatation (HSD).
Methods: Evaluation for associated conditions, prothrombotic disorders, and studies of hepatic perisinusoidal cell activation in consecutive patients, seen between 1993 and 2002, with isolated sinusoidal dilatation unrelated to outflow block, sinusoidal infiltration, or hepatic granulomas.
Results: Among 11 patients, associated conditions were prothrombotic disorders (n = 5) and oral contraceptive use (n = 3). Prothrombotic disorders were polycythemia vera (n = 1) and anticardiolipin antibodies combined with lupus anticoagulant (n = 4). No genetic thrombophilia factor was found. Of four patients with lupus anticoagulant, three had antinuclear factors and high serum levels of anticardiolipin antibodies at repeated testing. There was no evidence of intrahepatic or extrahepatic thrombosis in any of the patients. Sinusoidal dilatation was marked in six of 11 patients (54%), including two patients with antiphospholipid antibodies. Activated perisinusoidal cells were only found around markedly dilated sinusoids.
Conclusion: Idiopathic pure HSD is frequently associated with the immunological features of the antiphospholipid syndrome. Therefore, finding pure HSD in a liver biopsy specimen should prompt the search for antiphospholipid antibodies.